Uveal Diseases Umut Aslı Dinç., MD., FEBO Associate Professor in Ophthalmology Yeditepe University Eye Hospital

Uveal Tract Pigmented, vascular structure that lies between the sclera&retina Consists of: İris Ciliary body Choroid

Uveal Tract Supplies most of the ocular vasculature through the anterior and posterior ciliary branches of ophthalmic artery. Produces aqeous humor Controls accomodation at near Supplies aqeous outflow by trabecular meshwork

Uveitis Inflammation of the uveal tract and adjacent structures. Mostly the cause is unknown.

Uveitis Classification Anterior uveitis (iris and ciliary body) Iritis, anterior cyclitis, iridocyclitis Intermediate uveitis (pars plana of ciliary body, anterior vitreus and peripheral retina) Posterior cyclitis, pars planitis Posterior uveitis (choroid) Choroiditis, chorioretinits Panuveitis

Uveitis Classification Acute uveitis Chronic uveitis

Acute Uveitis-Clinical Features Pain Redness Photophia Epiphora Blurred vision Floaters

Chronic Uveitis-Clinical Features Fewer or none of the acute symptoms Periods of exacerbations and remissions

Causes of Uveitis Idiopathic Infectious (bacterial, viral, fungal, parasitic) Traumatic Post-surgery Tuberculosis Sarcoidosis Behçet’s disease Spondiloartropathies Inflammatory bowel diseases Collagen vascular diseases Medication

History taking in uveitis Present illness Onset, course, symptoms, laterality Past ocular history Previous episodes, treatment, ocular trauma or surgery Medical history Systemic disease (sarcoidosis, tuberculosis, syphilis, Juvenile rheumatoid artritis, AIDS, etc), maternal infection Sexual history, intravenous drug abuse Demograhic data Age, sex, race

History taking in uveitis Review of symptoms General-fever, weight loss, malaise, night sweats Rheumatologic-arthralgias, lower back pain, joint sitffness Dermatologic-rashes, sores, alopecia, vitiligo, poliosis, insect bites Neurologic-tinnitus, headache, meningism, paresthesias, weaksness/paralysis GIS- diarrhea, bloody stools, aphtous ulcers GUS-dysuria, discharge, genital ulcers, balanitis

Uveitis-Clinical Features Inflammatory cells in the anterior chamber Keratic precipitates on corneal endothelium Anterior synechiae (adhesions of iris to cornea) Posterior synechiae (adhesions of iris to lens) Inflammatory cells in the vitreous cavity (vitritis) Sheating of retinal vessels Optic disc or macular edema Choroidal or chorioretinal infiltrates

Anterior Uveitis Anterior segment cells Anterior segment flare

Anterior Uveitis Scleral injection Keratic precipitates on corneal endothelium

Anterior Uveitis Posterior synechiae

Anterior Uveitis Idiopathic Infectious (Herpetic uveitis, Bacteriel uveitis) Traumatic Fuch’s heterochromic iridocyclitis Immune-mediated Behçet’s disease Seronegative spondiloartropathies (Ankylosing Spondylitis, Reactive (Reiter) Arthritis, Psoriatic Arthritis ) Inflammatory bowel diseases (Crohn disease, ulcerative colitis) Juvenile Rheumatoid Arthritis Sarcoidosis Tuberculosis Toxic (Rifabutin, sulfonamides, cidofovir)

Idiopathic anterior uveitis Most common form of ocular inflammation No systemic or ocular cause Relief with topical steroids and cycloplegic drops

Fuch’s heterochromic iridocyclitis Unilateral Heterochromia (lighter iris color is typical) Vision loss secondary glaucoma and cataract

Fuch’s heterochromic iridocyclitis Heterochromia due to iris atrophy

Herpetic uveitis Keratitis Iris atrophy

Herpetic zoster uveitis

Behçet’s disease Key features Uveitis (anterior, posterior) Recurrent oral ulcers Recurrent genital ulcers Skin lesions Associated features Erythema nodosum Arthritis İntestinal ulcers Vascular lesions-thrombophlebitis, arteriel occlusions, aneurysms CNS involvement

Behçet’s disease HLA-B51 Pathergy test +

Seronegative spondiloartropathies HLA B27 + Sacroiliac joint radiography

Juvenile Rheumathoid Arthritis Most frequently in RF (-), ANA (+), oligoarticular type White eye Band keratopathy Posterior synechiae Cataract

Sarcoidosis Noncaseating granuloma Anterior and posterior uveitis Bilateral hilar lymphadenopathy Pulmonary parenchymal disease Chest radiography Serum ACE enzyme

Sarcoidosis Granulomatous reaction with iris nodules and mutton fat keratic precipitates

Tuberculosis Caseating granuloma Anterior and posterior uveitis Chronic inflammation PPD test Chest Radiography

Tuberculosis Granulomatous reaction with iris nodules and mutton fat keratic precipitates

Intermediate Uveitis Blurred vision and floaters Typically bilateral Vision loss secondary to cystoid macular edema Pars planitis... idiopathic type

Intermediate Uveitis Vitreous snowballs Snowbanking (exudation at pars plana)

Intermediate Uveitis Perivascular sheating Vitreous snowball

Posterior Uveitis Vitritis Choroiditis Retinitis Papillitis Retinal detachment

Posterior Uveitis Viral (CMV, VZV, HIV in immunodeficiency) Bacterial Fungal (candida albicans, histoplasma capsulatum, coccidioides immitis) Toxoplasmosis Parasitic (toxocariasis, cysticercosis, onchocerciasis) Syphilis Behçet’s disease Sarcoidosis Tuberculosis

Viral Posterior Uveitis CMV chorioretinitis

Viral Posterior Uveitis Acute retinal necrosis secondary to HSV infection

Viral Posterior Uveitis HIV chorioretinitis

Fungal Posterior Uveitis Ocular candidiasis

Toxoplasmosis Toxoplasma gondii Transmission by infected raw meat or congenitally via plasenta Recurrent chorioretinitis and severe vitritis Toxoplasma IgM, IgG and PCR

Congenital Toxoplasmosis Nonactive chorioretinal scars

Toxoplasmosis “Headlight in fog”

Toxocariasis Typical toxocara granuloma Traction of macula, optic disc Tx: steroids Severe inflammation when microorganism dies.

Sarcoidosis

Tuberculosis Choroidal tubercule

Behçet’s disease

Syphilis Usually in acquired syphilis VDRL, RPR, FTA-ABs+

Anti-inflammatory Therapy Corticosteroids Topical drops/ periocular injection/ systemic) Cytotoxic drugs Antimetabolites (azathioprine, methotrexate) Alkylating agents (cyclophoshamide, chlorambucil) Immunomodulator agents (cyclosporin, tacrolimus) Anti-TNF agents (etanercept, infliximab, adalimumab)

Complications of Uveitis Band keratopathy Posterior synechiae Cataract Glaucoma Cystoid macular edema Optic atrophy